Benign recurrent intrahepatic cholestasis (BRIC) is a rare cause of cholestasis in children, characterized by recurrent episodes of cholestasis without permanent liver damage. BRIC type 2 is an autosomal recessively inherited disorder caused by mutations in ABCB11 gene. A 6-year-old girl had recurrent episodes of jaundice and pruritus. At 2 months of her age, she had jaundice with hepatosplenomegaly. Liver function tests showed cholestatic hepatitis and low GGT level. Liver biopsy revealed diffuse giant cell transformation, ballooning degeneration, bile duct paucity, intracytoplasmic cholestasis, moderate portal inflammation, and periportal fibrosis. ABCB11 gene study revealed novel compound heterozygous mutations including c.2075+3A＞G in IVS17, and p.R1221K. Her liver function test was normalized at 12 months of age. At the age of 5 years, she developed abdominal pain, steatorrhea, jaundice and pruritus. Her liver function tests and pruritus improved after starting phenylbutyrate and rifampicin. The BRIC type 2 was diagnosed because of recurrent cholestasis and ABCB11 gene study. Her younger brother had jaundice at 2 months of age and his genetic test revealed the same mutations as his sister. This is the first report of BRIC 2 confirmed by ABCB11 gene study in Korea.